Abstract

This project is a modified continuation of the newborn screening study which was previously funded. Specific approaches of the current project include: 1. Identification, testing, counseling, and education of pregnant women who are at risk to be carriers of Hb S gene or other abnormal hemoglobins. Pregnant women at risk (Black, Hispanics, Asians,....) will be identified in our prenatal clinic and tested for the presence of hemoglobinopathies. Those that are carriers of Hb S gene or other abnormal hemoglobins will be followed in our Center for counseling and education. The spouses of these patients will also be tested and further counseling and education will ensue including the initiation of the process of prenatal diagnosis. 2. Counseling, education, and possible prenatal diagnosis of patients with sickle cell anemia who became pregnant. The spouses of these patients will also be tested as was mentioned above. 3. Confirmatory testing of newborns at our institution who are found to be at risk to have hemoglobinopathies by the State Newborn Screening Project.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Comprehensive Center (P60)
Project #
5P60HL038632-07
Application #
3758608
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
7
Fiscal Year
1994
Total Cost
Indirect Cost

  Institution

Name
Children's Hospital of Philadelphia
Department
Type
DUNS #
073757627
City
Philadelphia
State
PA
Country
United States
Zip Code
19104

  Publications

Ballas, Samir K; Connes, Philippe; Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia (2018) Rheological properties of sickle erythrocytes in patients with sickle-cell anemia: The effect of hydroxyurea, fetal hemoglobin, and ?-thalassemia. Eur J Haematol 101:798-803
Kwiatkowski, Janet L; Zimmerman, Robert A; Pollock, Avrum N et al. (2009) Silent infarcts in young children with sickle cell disease. Br J Haematol 146:300-5
Adachi, Kazuhiko; Ding, Min; Asakura, Toshio et al. (2009) Relationship between beta4 hydrogen bond and beta6 hydrophobic interactions during aggregate, fiber or crystal formation in oversaturated solutions of hemoglobin A and S. Arch Biochem Biophys 481:137-44
Kiryu, Shigeru; Sundaram, Tessa; Kubo, Shigeto et al. (2008) MRI assessment of lung parenchymal motion in normal mice and transgenic mice with sickle cell disease. J Magn Reson Imaging 27:49-56
Niebanck, Alison E; Pollock, Avrum N; Smith-Whitley, Kim et al. (2007) Headache in children with sickle cell disease: prevalence and associated factors. J Pediatr 151:67-72, 72.e1
Uematsu, Hidemasa; Takahashi, Masaya; Hatabu, Hiroto et al. (2007) Changes in T1 and T2 observed in brain magnetic resonance imaging with delivery of high concentrations of oxygen. J Comput Assist Tomogr 31:662-5
Obata, Kazuo; Mattiello, Julian; Asakura, Kenji et al. (2006) Exposure of blood from patients with sickle cell disease to air changes the morphological, oxygen-binding, and sickling properties of sickled erythrocytes. Am J Hematol 81:26-35
Akbar, Mohammed G K; Tamura, Yutaka; Ding, Min et al. (2006) Inhibition of hemoglobin S polymerization in vitro by a novel 15-mer EF-helix beta73 histidine-containing peptide. Biochemistry 45:8358-67
Adachi, Kazuhiko; Ding, Min; Surrey, Saul et al. (2006) The Hb A variant (beta73 Asp-->Leu) disrupts Hb S polymerization by a novel mechanism. J Mol Biol 362:528-38
Asakura, Toshio; Asakura, Kenji; Obata, Kazuo et al. (2005) Blood samples collected under venous oxygen pressure from patients with sickle cell disease contain a significant number of a new type of reversibly sickled cells: constancy of the percentage of sickled cells in individual patients during steady state. Am J Hematol 80:249-56

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